25 research outputs found

    Alveolar capillary dysplasia: A logical approach to a fatal disease

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    Purpose: The aim of the study was to review the authors' experience with alveolar capillary dysplasia (ACD), a cause of persistent pulmonary hypertension of the neonate (PPHN) caused by decreased alveolar units, dilated anomalous pulmonary veins, thick-walled arterioles, and thickened interalveolar septa. Methods: The records of all neonates with ACD were reviewed from Children's Hospital, Columbus, Ohio, and Sophia's Children's Hospital, Rotterdam, The Netherlands. The clinical characteristics and pathological findings are discussed. Results: Eight neonates were diagnosed with ACD from 1994 to 2002. Twenty-five percent (2/8) experienced respiratory distress immediately after birth, whereas 75% (6/8) had normal Apgar scores but deteriorated 1.5 hours to 30 days after birth. All infants required conventional ventilation initially; 50% (4/8) were placed on high-frequency oscillating ventilation and 87% (7/8) on extracorporeal membrane oxygenation. A premorbid diagnosis was established in 3 patients by open lung biopsy. The diagnosis of ACD was confirmed at autopsy in all patients. Conclusions: ACD is a fatal disease that should be suspected in all neonates with respiratory failure and PPHN who fail conventional therapy. Prompt diagnosis helps to avoid prolongation of costly treatment modalities in a uniformly fatal disease. An algorithm is proposed in which neonates with PPHN who fail treatment with extracorporeal membrane oxygenation are managed by open lung biopsy

    Simultaneous Robotic-Assisted Laparoscopy for Bladder and Bowel Reconstruction

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    © Copyright 2018, Mary Ann Liebert, Inc., publishers 2018. Introduction: Patients with neurogenic bladder frequently also have bowel dysfunction and a simultaneous urologic and colorectal reconstruction is possible. We present our experience with combined reconstructive procedures using robot-Assisted laparoscopy, and demonstrate the utility of a minimally invasive approach that considers both the bowel and bladder management of these patients. Methods: We retrospectively reviewed all patients who underwent combined bowel and urologic reconstruction at our institution since the start of our multidisciplinary robotic program. Results: Seven patients were identified in our cohort with a mean age of 6.4 years (3.8-10.1 years). Six patients had myelomeningocele and 1 had caudal regression. Malone appendicostomies were placed in all 7 patients. A split appendix technique was used as a conduit in 5 patients, in situ appendix in 1, and neoappendicostomy with cecal flap in 1. Six patients had a Mitrofanoff appendiceal conduit created, while 1 patient had a sigmoid colovesicostomy for urinary diversion. Five patients required bladder neck repair. One patient had stenosis of the Mitrofanoff and one patient had an anastomotic leak of the sigmoid anastomosis. The average operating time was 526 minutes (313-724 minutes). The median length of stay (LOS) was 5 days (4-7 days), excluding one outlier who suffered an anastomotic leak and had an extended LOS (50 days). All patients who underwent continent bladder reconstruction are dry on their current catheterizing regimen, 6/7 are clean with antegrade flushes. Conclusion: Patients with neurogenic bladder often have coexisting bowel dysfunction, which provides an opportunity to reconstruct both organ systems simultaneously and achieve social urinary and bowel continence. Before committing to any intervention, the surgeon should consider both the urologic and gastrointestinal needs of the patient, and perform the needed procedures simultaneously. We describe a number of combined operations aimed at bowel and bladder management that can be performed safely using robot-Assisted laparoscopy
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